Alia Al-Mohtaseb, MD FRCPath King Abdulla University Hospital - PowerPoint PPT Presentation

Alia Al-Mohtaseb, MD FRCPath King Abdulla University Hospital Jordan University of S cience and Technology

 Case history…  Microscopic images…  Differential diagnosis…  Diagnosis.  Prognosis and clinical outcome.  Literature review.

 A 42 year old female patient, HTN, DM, P6.  Presented with inguinal pain.  Found to have a vulval mass, measuring 3.5 x 3.5 cm.  Left hemivulvectomy.

Foci of perineural invasion are seen.

CD117- Negative P63 collagen

 Left groin mass and pain.  Left vulvar mass.  S ignificantly enlarged left inguinal lymph nodes with necrotic centre.  Multiple bilateral innumerable pulmonary nodules were noted. Left vulvectomy and left groin dissection…  p T1aN1b  Recurrent tumor 1.5cm  4 cycles of chemotherapy 

 First documented in 1864  Often diagnosed at advanced stage due to late presentation and low clinical suspicion  Mean age at diagnosis: 60 years (range 33 - 93 years)  Constitutes approximately 2 - 7% of vulvar and less than 1% of gynecologic malignancies

 Diagnostic criteria:  Compatible with origin from Bartholin gland, deep to the labia  Intact overlying skin  Transition between normal glandular tissue and carcinoma  No evidence of primary tumor elsewhere

• Posterolateral to labium maj us, involving the lower part of the vulva • S low growing, painless, palpable or visible tumor posterior to the labium maj us • Rarely, patients may experience rectal or vaginal pain and discomfort, bleeding (postcoital), dyspareunia and pruritus

 S quamous cell carcinomas (S CC).  Adenocarcinomas.  Adenoid cystic carcinoma (ACC, ~15% )  Other histological types (~5% ) include:  Transitional cell carcinoma, adenosquamous carcinoma, poorly differentiated carcinoma, low grade epithelial - myoepithelial carcinoma, sarcoma, melanoma and clear cell carcinoma

 May originate from myoepithelial cells  Tumor cells usually have low cytologic grade and are arranged in a cribriform pattern and the (pseudo) lumens are filled with mucin or hyalinized basement membrane material  Frequent local recurrence

 CEA.  CD117.  PASD.  S100 , SMA and p63.

 Bartholin gland cyst.  Inflammatory mass.  Endometriosis.  Angiomyofibroblastoma.

 S tage of the disease at presentation.

 Risk factors are still unclear.  The symptoms are usually non-specific.  There is currently no consensus regarding the optimal surgical treatment and the question whether to do or not a systematic inguinal femoral lymph node dissection is still controversial.  Guidelines for postoperative chemotherapy or chemoradiotherapy are not established, despite the relative frequency of microscopically positive surgical resection margin.

 The most frequent metastatic site is the lung Bernstein et al., noted that 5 of 20 patients died from lung metastasis in an interval varying from 4 to 23 years after initial treatment.  Brain metastasis are also described in the literature.

 The adenoid cystic carcinoma of the Bartholin’s gland: a literature review; Antonio Cassio Assis Pellizzon-Brazil; Pellizzon Applied Cancer Research (2018).  Bernstein S G, Voet RL, Lifshitz S , Buchsbaum HJ. Adenoid cystic carcinoma of Bartholin's gland. Case report and review of the literature. Am J Obstet Gynecol. 1983.  WHO classification of the tumours of female reproductive organs, 2014.  Akbarzadeh-Jahromi M, S ari Aslani F , Omidifar N, Amooee S . Adenoid Cystic Carcinoma of Bartholin’s Gland Clinically Mimics Endometriosis, A Case Report. Iran J Med S ci. 2014